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Ctexli (chenodiol) — Blue Cross Blue Shield of Alabama

cerebrotendinous xanthomatosis (CTX)

Initial criteria

  • ONE of the following:
  • The requested agent is eligible for continuation of therapy AND ONE of the following:
  • - The patient has been treated with the requested agent (starting on samples is not approvable) within the past 90 days OR
  • - The prescriber states the patient has been treated with the requested agent (starting on samples is not approvable) within the past 90 days AND is at risk if therapy is changed
  • OR BOTH of the following:
  • ONE of the following:
  • - The patient has a diagnosis of cerebrotendinous xanthomatosis (CTX) as confirmed by ONE of the following:
  • • Genetic testing confirming variants in the CYP27A1 gene OR
  • • ALL of the following:
  • – Elevated plasma cholestanol ≥5 to 10 times ULN (upper limit of normal)
  • – Urine positive for bile alcohols
  • – Clinical findings consistent with CTX (e.g., xanthomas in lungs, tendons, bone or central nervous system; infantile-onset diarrhea; childhood-onset cataracts; adult-onset progressive neurologic dysfunction including dementia, psychiatric disturbances, pyramidal and/or cerebellar signs, dystonia, atypical parkinsonism, peripheral neuropathy, and seizures)
  • OR
  • - The patient has another FDA labeled indication for the requested agent and route of administration
  • AND if the patient has an FDA labeled indication, then ONE of the following:
  • • The patient’s age is within FDA labeling for the requested indication for the requested agent OR
  • • There is support for using the requested agent for the patient’s age for the requested indication
  • AND
  • - The patient has had baseline liver transaminase (ALT, AST) and total bilirubin levels prior to initiating the requested agent
  • - The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist, geneticist, hepatologist, endocrinologist, neurologist), or the prescriber has consulted with such a specialist
  • - The patient does NOT have any FDA labeled contraindications to the requested agent

Reauthorization criteria

  • The patient has been previously approved for the requested agent through the plan’s Prior Authorization process
  • The patient has had clinical benefit with the requested agent
  • The patient is monitored for changes in liver transaminase (ALT, AST) and total bilirubin levels AND BOTH of the following:
  • - Liver transaminase levels <3× ULN
  • - Total bilirubin level <2× ULN
  • The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist, geneticist, hepatologist, endocrinologist, neurologist), or has consulted with such a specialist
  • The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

12 months